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Paediatrics RACP Exam Study > Paediatric Haematology > Flashcards

Paediatric Haematology Flashcards

1
Q

What is the life span of:

  • neutrophils?
  • platelets?
  • red blood cells?
A
  • neutrophils 6-8hrs
  • platelets 10 days
  • red cells 120 days
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2
Q

Where are the main sites and stages of haematopoesis in embryonic/foetal life?

A
  • Mesoblastic stage → occurs in extra-embryonic structures e.g. yolk sac/placenta from 2-12/40
  • Hepatic stage → predominant blood production site from 6-24/40, ↓ in 2nd trimester, 85% erythroid (no neutrophils)
  • Myeloid stage from 24/40 onwards, 40% erythroid (15% neutrophils)
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3
Q

What happens to red cell, neutrophil, and platelet size/numbers during foetal life?

A

Red cells - higher MCV and MCH but lower Hb and Hct 22/40 - at term, MCV/MCH lower and Hb/Hct higher
Platelets - same size, but numbers gradually increase from 22/40
Neutrophils - none before 2nd trimester and scarce in blood until 3rd. Lots of progenitor cells in blood.

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4
Q

What is the Kleihauer-Betke test?

A

Acid elution test

Determines if fetal RBCs in maternal circulation

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5
Q

Describe the clinical phenotype of Fanconi anaemia:

A
  • Abnormal thumbs, absent radii, short stature
  • skin hyperpigmentation e.g. café au lait spots
  • abnormal facial features (triangular face, microcephaly)
  • abnormal development
  • marrow failure
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6
Q

What is the main cancer that people with Fanconi anaemia get?

A
  • AML

- Bone marrow failure 80% by age 20

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7
Q

Describe the clinical phenotype of Diamond-Blackfan anaemia:

A
  • anaemic, pale neonate/infant (75% by 6/12)
  • short stature with craniofacial abnormalities (e.g. cleft lip, hypertelorism, micrognathia), web neck
  • triphalangeal thumb (classical)
  • absent/abnormal kidney e.g. horseshoe (25% have abnormal phenotype)
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8
Q

What are the laboratory findings in Diamond-Blackfan anaemia:

A
  • normochromic macrocytic anaemia
  • low reticulocytes
  • thrombocytosis (rare: thrombocytopenia)
  • marrow normally cellular aside from RBC precursors
  • diagnostically: erythrocyte adenosine deaminase (ADA) activity is increased
  • generally RBCs only affected line
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9
Q

What cancers do you get with Diamond-Blackfan?

A
  • acute myelogenous leukemia
  • myelodysplastic syndrome
  • female genital Ca
  • osteosarcoma
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10
Q

What is activated protein C resistance associated with?

A
  • Factor V Leiden mutation
  • inability of protein C to cleave factor Va and VIIIa.
  • longer duration of thrombin degradation, hypercoagulable state
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11
Q

What are the clotting pathways assessed by APTT?

A
  • Intrinsic coagulation pathway (factors XII, XI, IX, VIII)

- Final common pathway (factors II, V, X and fibrinogen).

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12
Q

What areas is G6PD defect common in?

A
  • Mediterranean region (esp Greece, Italy)
  • Philippines
  • China
  • Africa
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13
Q

What are common triggers for G6PD?

A
  • Fava beans
  • nitrofurantoin
  • co-trimoxazole
  • primaquine
  • chloraquine
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14
Q

What is the typical presentation of LCH?

A
  • Presents with osteolytic bone lesions +/- multi-organ involvement
  • LCH cells can infiltrate most body tissues (except kidney and heart)
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15
Q

Histiocyte markers CD1a, S100, and CD207

(langerin) makes you think…

A

Langerhans Cell Histiocytosis

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16
Q

Birbeck granule – a tennis racket shaped granule in the cytoplasm of cells on electron microscopy is associated with…

A

Langerhans Cell Histiocytosis

17
Q

Presentation: Short with skeletal dysplasia, eczema, learning difficulties, poor feeding/FTT/steatorrhoea, may have increased infections (mild neutropenia +/- low Hb +/- low platelets)

A

Schwachman-Diamond Syndrome (exocrine pancreatic insufficiency, bone marrow dysfunction, skeletal abnormalities and short stature)

18
Q

What is in cryoprecipitate?

A
  • factor VIII
  • fibrinogen
  • vWF
  • factor XIII
19
Q

What is the most common cause of familial thrombophilia (deep vein thrombosis and pulmonary emboli)?

A

Activated protein C resistance/Factor V Leiden (white population)

20
Q

Why might you get low haptoglobin?

A
  • Intravascular Haemolysis
  • Wilsons

(Haptoglobin: acute phase reactant, mops up free haemoglobin from outside RBCs to stop it being toxic)

21
Q

What are the most important inhibitors of the coagulation cascade?

A
  • Antithrombin
  • Protein C
  • Protein S
22
Q

What are the different types of vW disease?

A

Type 1: low levels of vWF, most common ~70%
Type 2: change in vWF function, range of defects
Type 3: complete deficiency of vWF

23
Q

What is the typical skeletal anomaly with Diamond-Blackfan?

A
  • triphalangeal thumb
24
Q

What is the typical skeletal anomaly with Fanconi anaemia?

A
  • abnormal thumbs e.g extra/duplicate, absent, dangling

- absent radii

25
Q

What is foetal haemoglobin (HbF) comprised of?

A

2 alpha chains and 2 gamma chains

26
Q

What is HbA2 (the other adult haemoglobin) comprised of?

A

2 alpha chains and 2 delta chains

27
Q

Which infective gastroenteritis is associated with iron malabsorption?

A

Giardia lamblia infection

28
Q

What is the diagnostic test for hereditary spherocytosis?

A

EMA binding test

29
Q

What class of antibody is warm antibody autoimmune haemolysis?

A

IgG

30
Q

What class of antibody is cold antibody autoimmune haemolysis?

A

IgM, anti I/i

31
Q

What is the most common drug causing autoimmune haemolysis?

A

Cephalosporins

The most common cause overall is probably post infectious (e.g. mycoplasma, EBV)

32
Q

What cancer do you worry about with bone marrow failure syndromes?

A

AML for all of them
Fanconi anaemia & dyskeratosis congenita give you SCC
Diamond-Blackfan - sarcoma

33
Q

What is the half-life of the vitamin K dependent factors?

A 
VII - 4-6 hours
IX - 24 hours
X - 24-48 hours
II - 60 hours
Protein C - 8 hours
Protein S - 30 hours
34
Q

In what condition(s) do you see target cells on the blood film?

A

Thalassemias

35
Q

In what condition(s) do you see spherocytes on the blood film?

A

Hereditary spherocytosis

Autoimmune haemolytic anaemia

36
Q

What is the most likely coagulopathy? 4/12 male, significant oozing from wound following talipes repair. Breastfed, well, no PMH/FHx bleeding.
Hb 45 g/L [95-140], PT-INR 1.2 [1.0-1.4], APTT 120

A

Factor VIII deficiency/haemophilia A

37
Q

What helps distinguish intra/extravascular haemolysis?

A

Urinary haemosiderin

38
Q

Signs of intravascular haemolysis?

A
  • low haptoglobin
  • urinary haemosiderin/haemoglobin
  • high LDH

Paediatrics RACP Exam Study (30 decks)

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